Adult-onset Still's disease: an unusual presentation of rubella infection

S.H.K. HUANG, MD; W.E. DECOTEAU, MD

Still's disease in the adult is uncom- and tenderness of both ankles and sion, but it rose to 1/5120 1 mon."4 Recently Harth, Thompson several metacarpophalangeal joints. week later and 1/10240 6 weeks and Ralph4 described four adults The leukocyte count was 25.75 x later. Roentgenograms of the joints with this disease. Two of the pa- 109/1 (78% neutrophils), the ery- were normal. A technetium 99m tients had elevated rubella antibody throcyte sedimentation rate (ESR) liver and spleen scan revealed he- titres, and rubella virus was isolated 91 mm/h (Wintrobe) and the C- patosplenomegaly, with an increase from the urine of one of them. In reactive protein value 4+. She was in the splenic span to 20 cm. An an accompanying editorial5 Petty treated with acetylsalicylic acid, 6 echocardiogram, gallium 67 total noted the "provocative and possibly g/d and penicillin G, 500 mg four body scan, abdominal ultrasono- coincidental similarities" between times a day by mouth for, tenta- gram, chest roentgenogram and rubella arthritis and Still's disease, tively, rheumatic fever. electrocardiogram were all normal. and suggested further studies to When there was no improvement The patient's temperature con- clarify the relation between rubella a week later the woman was ad- tinued to spike to 40°C each day infection and Still's disease. There- mitted to University Hospital. By in association with chills and pro- fore, we are reporting the case of this time she had lost 16 kg. She fuse sweating. Typically the rash a 29-year-old woman with typical looked ill, had a temperature of faded in the evening and recurred adult-onset Still's disease who had 39°C and was having chills. A the next day with the fever. a brisk rise in the rubella antibody salmon-pink macular rash was Four days after admission acute titre. noted over the extremities and the monoarthritis of the left wrist was trunk. A grade 3/6 systolic ejec- noted. Aspirated synovial fluid had Case report tion murmur was heard at the left a leukocyte count of 40 x 10'/l A 29-year-old woman presented sternal border. The liver and spleen (93% neutrophils); a routine cul- to her family physician with a sore were palpable, but there was no ture was negative. The inflamma- throat, fever, rash, polyarthralgia synovitis. tion spontaneously subsided in 48 and severe myalgia of the back and The hemoglobin level was 12 hours but was followed 4 days later legs. Her fever was remittent, with g/dl, the, leukocyte count 9.2 x by acute arthritis of the right knee. a daily spike in temperature to 109/l, the ESR 105 mm/h (Win- Shortly thereafter the left fifth prox- 40°C associated with chills and trobe) and the serum albumin level imal interphalangeal joint became profuse sweating. The rash con- 2.9 g/dl. The serum levels of the involved. Therapy with indome- sisted of small, mildly pruritic, sal- following were persistently elev- thacin, 50 mg three times a day, mon-pink macules over the legs ated: alkaline phosphatase, at 110 was begun; almost immediately the and, later, the back and arms. to 234 (normal 30 to 85) IU/l; daily temperature spike was re- Characteristically, the rash disap- lactate dehydrogenase, at 272 to duced and the rash cleared. peared during the night and reap- 386 (normal 100 to 270) IU/l; and The patient was discharged feel- peared the next day with the fever. glutamic oxaloacetic transaminase, ing well following a total stay in The joint pain was migratory, af- at 57 to 109 (normal 10 to 50) hospital of 16 days. Follow-up fecting the wrists, elbows, knees, IU/l. The following gave either visits 1 and 2 months later revealed ankles and, finally, the small joints negative or normal results: tests for no signs or symptoms, and the labo- of the hands and feet. antinuclear antibody, DNA binding ratory findings were all normal ex- At the time of physical examina- and rheumatoid factor; measure- cept for the rubella antibody titre, tion her temperature was 40°C and ment of the serum levels of immu- which had remained at 1/ 10240. the pulse rate 100 beats/min. A 1/6 noglobulins A, G and M and Discussion systolic ejection murmur was de- third component of complement; tected along the left sternal border. multiple blood and urine cultures; The patient we have presented There was swelling of both knees and serologic tests for cytomegalo- had typical clinical features of virus, measles and mumps viruses, adult-onset Still's disease. In addi- Mycoplasma, Salmonella, hepatitis tion, there was strong evidence of From the rheumatic disease unit, recent rubella infection because of department of medicine, University B antigen, Brucel a, Toxoplasma, Hospital, Saskatoon Yersinia, Treponema pallidum, he- a brisk rise in the titre of rubella terophil antibody and antistrepto- hemagglutination-inhibiting anti- Reprint requests to: Dr S.H.K. Huang, Rheumatic disease unit, Department lysin 0. The titre of rubella he- body. of medicine, University Hospital, magglutination-inhibiting antibody The relation between the pa- Saskatoon, Sask. S7N OXO was 1/1280 at the time of admis- tient's Still's disease and rubella in- CMA JOURNAL/JUNE 7, 1980/VOL. 122 1275 fection is unknown, but some of pincott, Philadelphia, 1977, p 579 1157, 1975 the possibilities are as follows. 9. OGRA PL, HERD JK: Serologic asso- 11. COOPER LZ: The rubella hemagglu- ciation of rubella virus infection and tination inhibition test, in Rubella; First, rubella infection can produce juvenile rheumatoid arthritis (abstr). First Annual Symposium of the a variety of musculoskeletal disor- Arthritis Rheum 15: 121, 1972 Eastern Pennsylvania Branch, Amer- ders, including polyarthritis of the 10. OGRA PL, OGRA SS, CHIBA Y, et al: ican Society for Microbiology, small joints, which closely mimics Rubella-virus infection in juvenile FRIEDMAN H, PRIER JE (eds), CC adult-onset rheumatoid arthritis,4 rheumatoid arthritis. Lancet 1: Thomas, Springfield, III, 1973, p 8 an illness that resembles juvenile rheumatoid arthritis,9'0 and, as in our case, a systemic illness that has I BOOKS the typical features of adult-onset Still's disease. Second, it is possible continued from page 1274 that the rubella antibody response in this patient was nonspecific, as HORMONAL BIOLOGY OF ENDOME- PRACTICAL OBSTETRICS PROBLEMS. patients with various inflammatory TRIAL CANCER. A Series of Work- 5th ed. Ian Donald. 1071 pp. lllust. Lloyd- disorders can show nonspecific in- shops on the Biology of Human Cancer. Luke (Medical Books) Ltd., London, Report No. 8. UICC Technical Report 1979. Price not stated. ISBN 0-85324- creases in the titres of various anti- Series - Volume 42. Edited by G.S. 132-5 bodies.11 However, the eightfold Richardson and D.T. MacLaughlin. 187 MED- rise in pp. Illust. International Union Against PROBLEM SOLVING IN CLINICAL the rubella antibody titre Cancer, Geneva, 1978. $13.41, paper- ICINE. From Data to Diagnosis. Paul and the lack of elevation of other bound. ISBN 92-9018-042-0 Cutier. 373 pp. The Williams & Wilkins in Company, Baltimore; the Macmillan antibody titres our patient make A MANUAL OF ADVERSE DRUG INTER- Company of Canada Limited, Toronto, this unlikely. Finally, it is possible, ACTIONS. 2nd ed. J.P. Griffin and P.F. 1979. $24.50. ISBN 0-683-0225-2 though unlikely, that the concomit- D'Arcy. 402 pp. Illust. John Wright & PROSTATIC CANCER. Edited by Gerald ant occurrence of adult-onset Still's Sons Ltd., Bristol; Year Book Medical P. Murphy. 238 pp. lllust. PSG Publish- Publishers Inc., Chicago, 1979. Price ing Company, Inc., Littleton, Massa- disease and rubella infection in our not stated. ISBN 0-8151-4001-0 chusetts; the Macmillan Company of patient was purely coincidental. Canada Limited, Toronto, 1979. $30.95. MEDICAL EXPERIMENTATION. Edited ISBN 0-88416-190-0 Unfortunately, no attempt was by Amnon Carmi. 148 pp. Turtledove made to isolate rubella virus from Publishing, Ramat Gan, Israel; ISBS, PSYCHO-SOCIAL ASPECTS OF A SE- the synovial aspirate, which might Inc., Forest Grove, Oregon, 1979. $12.50. VERE BURN: A Review of the Litera- ISBN 965-20-0002-7 ture. 1979 Supplement to the Interna- have clarified the association be- tional Bibliography on Burns. M.L. tween these two entities. However, MEDICAL ONCOLOGY. An Advanced Bowden, Claudella A. Jones and Irving this report should alert clinicians Course. A Self-Assessment Guide for Feller. 127 pp. lllust. National Institute Subspecialty Board Examinations and for Burn Medicine, Ann Arbor, Michi- to study their patients with adult- Practice. Joseph G. Sinkovics. 679 pp. gan, 1979. Price not stated, paperbound. onset Still's disease for the presence Illust. Marcel Dekker, Inc., New York, ISBN 0-917478-24-7 1979. $49.50. ISBN 0-8247-6863-9 of rubella and other infections. Like PULMONARY EDEMA. Edited by Alfred many other diseases, adult-onset MEMBRANE BIOENERGETICS. Based on P. Fishman and Eugene M. Renkin. Still's disease be a the International Workshop held at 261 pp. Illust. American Physiological may syndrome Cranbrook Schools, Bloomfield Hills, Society, Bethesda, Maryland; the Mac- of many causes. Michigan, July 5-7, 1979, in honor of millan Company of Canada Limited, Efraim Racker. Edited by C.P. Lee, G. Toronto, 1979. $38.95. ISBN 0-683-08245- Schatz and L. Ernster. 609 pp. Illust. 3 References Addison-Wesley Publishing Company, Inc., Reading, Massachusetts, 1979. PULMONARY EMBOLISM. Walter G. 1. BYWATERS EGL: Still's disease in $25.50, paperbound. ISBN 0-201-03999-0 Wolfe and David C. Sabiston, Jr. Vol- the adult. Ann Rheum Dis 30: 121, ume XXV in the series Major Problems 1971 OPHTHALMOLOGY. The Essentials. in Clinical Surgery. Edited by Paul A. 2. BUJAK JS, APTEKAR RG, DECKER David Miller. 290 pp. Illust. Houghton Ebert. 180 pp. Illust. W.B. Saunders JL, et al: Juvenile rheumatoid ar- Mifflin Professional Publishers, Boston; Company Canada, Ltd., Toronto, 1980. the Macmillan Company of Canada $16.80. ISBN 0-7216-9584-1 thritis presenting in the adult as Limited, Toronto, 1979. $22.50, paper- fever of unknown origin. Medicine bound. ISBN 0-89289-325-7 RADIONUCLIDE TECHNIOUES IN MED- (Baltimore) 52: 431, 1973 ICINE. Joan M. McAlister. 229 pp. lIlust. 3. APTEKAR RG, DECKER JL, BUJAK ORTHOPAEDICS IN PRIMARY CARE. Cambridge University Press, New York, JS, et al: Adult onset juvenile rheu- Chinni Pennathur Ramamurti. Edited by 1979. $23.50, clothbound; $10.95 paper- Richard Vernon Tinker. 385 pp. lllust. bound. ISBN 0-521-22402-0, clothbound; matoid arthritis. A rthritis Rheum The Williams & Wilkins Company, Balti- ISBN 0-521-29474-6, paperbound. 16: 715, 1973 more; the Macmillan Company of Can- 4. HARTH M, THOMPSON JM, RALPH ada Limited, Toronto, 1979. $46.95. REGULATING HEALTH CARE: THE ED: Adult-onset Still's disease. Can ISBN 0-683-07150-5 STRUGGLE FOR CONTROL. Proceed- Med Assoc J 120: 1507, 1979 ings of The Academy of Political 5. PETTY RE: Making the diagnosis of PAEDIATRIC ORTHOPAEDICS AND Science. Volume 33, Number 4. Edited FRACTURES. Volumes 1 and 2. 2nd ed. by Arthur Levin. 244 pp. The Academy Still's disease (E). Ibid, p 1480 W.J.W. Sharrard. 1752 pp. Illust. Black- of Political Science, New York, 1980. 6. KANTOR TG: Viral forms of ar- well Scientific Publications, London; $6.50, paperbound. ISSN 0065-0684 thritis. Mod Treat 6: 1146, 1969 the C.V. Mosby Company, Saint Louis, 7. JOHNSON RF, HALL AP: Rubella 1979. Price not stated. ISBN 0-632- THE SELECTION OF ESSENTIAL arthritis: report of cases studied by 00056-2 DRUGS. Second Report of the WHO latex tests. N Engl J Med 258: 743, Expert Committee. Technical Report PLASTIC SURGERY. 3rd ed. Edited by Series 641. 44 pp. World Health Organ- 1958 William C. Grabb and James W. Smith. ization, Geneva, 1979. $2.04, paper- 8. SCHMID FR: Infectious arthritis, in 591 pp. lllust. Little, Brown and Com- bound. ISBN 92-4-120641-1 Rheumatic Diseases: Diagnosis and pany (Inc.), Boston, 1979. Price not Management, KATZ WA (ed), Lip- stated, paperbound. ISBN 0-316-32268-7 continued on page 1283 1276 CMA JOURNAL/JUNE 7, 1980/VOL. 122